Tumor tritón maligno en región mandibular y cervical / Malignant triton tumor in the mandibular and cervical region

Introducción: El tumor tritón maligno es una neoplasia rara en la que se encuentran células rabdomioblásticas en un tumor maligno de la vaina de nervios periféricos, que se caracteriza por su agresividad y mal pronóstico. La localización en la cabeza y el cuello es poco frecuente. La inmunohistoquímica juega un papel importante en el diagnóstico. Objetivo: Describir un tumor tritón maligno de tamaño inusual. Presentación del caso: Paciente femenino, de 16 años, es referida al servicio de cirugía maxilofacial del Instituto Nacional de Pediatría, Ciudad de México, con un diagnóstico de tumor neuroectodérmico en región facial y cervical de un año de evolución. Clínicamente el tumor era exofítico, multilobulado, con zonas extensas de necrosis, superficie de varias tonalidades y un tamaño aproximado de 18 x 10 x 12 cm. Se realizó una biopsia e inmunohistoquímica que confirmó el diagnóstico de tumor tritón maligno. La paciente fue intervenida quirúrgicamente, procedimiento con el cual se eliminó totalmente la lesión, con márgenes de seguridad. La paciente presentó una evolución tórpida, con desenlace fatal al cabo de seis meses del tratamiento. Conclusiones: El tumor tritón es una neoplasia agresiva y su detección oportuna orienta al cirujano a ofrecer al paciente un tratamiento adecuado(AU)

Introduction: Malignant triton tumor is a rare neoplasm in which rhabdomyoblasts are present in a malignant tumor of the peripheral nerve sheath. This condition is characterized by its aggressiveness and bad prognosis. Location in the head and neck is infrequent. Immunohistochemical testing plays an important role in its diagnosis. Objective: Describe an unusually large malignant triton tumor. Case presentation: A case is presented of a female 16-year-old patient referred to the maxillofacial surgery service of the National Institute of Pediatrics in Mexico City with a diagnosis of neuroectodermal tumor of one year's evolution in the facial and cervical region. In clinical terms, the tumor was exophytic, multilobed, with extensive areas of necrosis, a surface in several shades of color and an approximate size of 18 x 10 x 12 cm. Biopsy and immunohistochemical testing confirmed the diagnosis of malignant triton tumor. The patient underwent surgery in which the lesion was totally excised with a safety margin. Evolution was clumsy, with a fatal outcome at six months of treatment. Conclusions: Triton tumor is an aggressive neoplasm whose early detection makes it possible for surgeons to provide an appropriate treatment(AU)

Clinicopathological analysis of malignant triton tumor of the posterior mediastinum / 上海第二医科大学学报

Objective To investigate the pathologic appearances,immunohistochemical features,and genetic changes of malignant triton tumor(MTT). Methods One case of MTT was studied pathologically and immunohistochemically,and the related literatures were reviewed. Results A huge mass,demonstrated in the thorax by X ray and CT scan was seen in the posterior mediastinum in the surgery.Histologically,the tumor was composed of spindle cells with significant atypia.Some of the tumor cells had dense eosinophilic cytoplasm.Immunohistochemical staining revealed positive for myoglobin,desmin and S-100 in most of the tumor cells.The pathological diagnosis was MTT of the posterior mediastinum. Conclusion Cases of MTT in the mediastinum are very rare,with less specific clinical and imaging manifestations.The diagnosis is mainly made on the basis of pathological examination and immunohistochemical staining.

CT and MRI Manifestations of Primary Malignant Triton Tumor:A Report of 3 Cases with Review of the Literature / 实用放射学杂志

Objective To improve the understanding of malignant triton tumor on imaging findings.Methods CT and MRI findings of rare malignant triton tumor in 3 cases confirmed histologically were analysed with review of the literatures.Results 3 tumors included prostatic malignant triton tumor with pulmonary metastatic tumors and neurofibromatosis-I in 1 case,left haunch malignant triton tumor in 1 case and right maxillary sinus malignant triton tumor with destruction of maxillary sinus paries in 1 case.The imaging appearances of tumors were the huge soft tissue mass which strongly suggested malignant tumors,high signal intensity on T2WI,circular septa inside the tumors with low signal intensity.Conclusion Although the qualitative diagnosis of malignant triton tumor depends on pathological examination,CT and MRI play a useful role in diagnosis and differential diagnosis of this tumors.

A Case of Malignant Triton Tumor In Nasal Cavity / 대한이비인후과학회지

Malignant triton tumor is a rare and usually aggressive sarcoma consisting of a malignant schwannoma with rhabdomyoblastic differentiation. Although this tumor is usually found at the extremities, 20% of cases are located in the head and neck region. This unusual tumor has not yet been described in the Korean Journal of Otolaryngology-Head and Neck Surgery. Malignant triton tumor is histologically high-grade, with large numbers of mitoses, prominent necrosis, and cellular pleomorphism. The clinical course for malignant triton tumor is usually one of rapid growth, early metastasis, and poor outcome in spite of therapy. Recently we experienced a case of malignant triton tumor, which involved the nasal cavity, maxillary and ethmoid sinus. The malignant tumor was treated with wide surgical excision followed by radiation therapy.